Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue

G. Bisogno, M. Carli, M. Stevens, O. Oberlin, J. Treuner, G. Scarzello, R. Colombatti, L. De Zen, C. R. Pinkerton

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Abstract

The MMT4 study was designed to explore an intensive chemotherapy regimen (MMT4-89) and the role of high-dose melphalan (MMT4-91) in children with metastatic soft tissue sarcoma, including extraosseous peripheral neuroectodermal tumor (PNET). Thirty-one patients with PNET were treated between 1989 and 1995 (11 according to MMT4-89 and 20 according to MMT4-91). Chemotherapy consisted of four CEVAIE cycles, each including three 3-week courses: CEV (carboplatin 500 mg/m2, epirubicin 150 mg/m2, vincristine 1.5 mg/m2), IVA ifosfamide 9 g/m2, actinomycin 1.5 mg/m2, vincristine 1.5 mg/m2), IVE (ifosfamide 9 g/m2, etoposide 600 mg/m2, vincristine 1.5 mg/m2). In MMT4-91 the fourth CEVAIE was replaced with melphalan 200 mg/m2 with stem cell rescue. The CEV combination was evaluated as a window study. Surgery followed the second cycle. Radiotherapy was administered to post-surgical residual disease. The response rate was 55% after CEV, rising to 80% after the first CEVAIE. Twenty-five patients achieved complete remission (CR). Overall, the 5-year EFS was 22.6%: 36.4% and 15% for patients treated according to MMT4-89 and MMT4-91, respectively (P = 0.3). Local control was achieved in 77% of irradiated patients vs 45% of non-irradiated. Age >10 years was associated with significantly poorer outcome (P = 0.04). In conclusion, despite the high CR rate, intensive chemotherapy with or without high-dose melphalan appeared to have little impact on the survival of patients with metastatic extraosseus PNET.

Original languageEnglish
Pages (from-to)297-302
Number of pages6
JournalBone Marrow Transplantation
Volume30
Issue number5
DOIs
Publication statusPublished - Sep 2002

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Keywords

  • Ewing
  • Megatherapy
  • Melphalan
  • Peripheral neuroectodermal tumor

ASJC Scopus subject areas

  • Hematology
  • Transplantation

Cite this

Bisogno, G., Carli, M., Stevens, M., Oberlin, O., Treuner, J., Scarzello, G., Colombatti, R., De Zen, L., & Pinkerton, C. R. (2002). Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue. Bone Marrow Transplantation, 30(5), 297-302. https://doi.org/10.1038/sj.bmt.1703617